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Optimal target blood pressure to reduce adverse cardiac remodelling in children with chronic kidney disease is uncertain. We hypothesised that lower blood pressure would reduce adverse cardiac remodelling.HOT-KID, a parallel-group, open-label, multicentre, randomised, controlled trial, was done in 14 clinical centres across England and Scotland. We included children aged 2-15 years with stage 1-4 chronic kidney disease-ie, an estimated glomerular filtration rate (eGFR) higher than 15 mL/min per 1·73 mBetween Oct 30, 2012, and Jan 5, 2017, 64 participants were randomly assigned to the intensive treatment group and 60 to the standard treatment group (median age of participants was 10·0 years [IQR 6·8-12·6], 69 [56%] were male and 107 [86%] were of white ethnicity). Median follow-up was 38·7 months (IQR 28·1-52·2). Blood pressure was lower in the intensive treatment group compared with standard treatment group (mean systolic pressure lower by 4 mm Hg, p=0·0012) but in both groups was close to the 50th percentile. The mean annual reduction in LVMI was similar for intensive and standard treatments (-1·9 g/mThese results suggest that cardiac remodelling in children with chronic kidney disease is related to blood pressure control and that a target office systolic blood pressure at the 50th percentile is close to the optimal target for preventing increased left ventricular mass.British Heart Foundation.

BACKGROUND Studies show that young people (YP) with chronic kidney disease do less well than their well peers in terms of mental health and employability. In the UK 30% of YP on renal replacement therapy have mental health issues compared to 15% of the general population and more are unemployed compared with their well peers. Many of the issues start in childhood as studies show that children with chronic kidney disease have significant psychosocial issues including: To address these issues, we developed the PEEER project to help improve Patient Empowerment, self-Esteem, Employability and Resilience. Pre-coronavirus disease (COVID-19) PEEER youth worker support was delivered face-to-face (F2F) in a 1:1 setting or group sessions in the hospital, youth spaces, or educational settings. With the onset of lockdown due to the COVID-19 pandemic from 23 March 2020 PEEER youth worker support was adapted to be delivered virtually on the ZOOM platform to meet the needs of CYP. AIMS To assess the effectiveness of PEEER youth work project in meeting the psychosocial needs of children and young people with kidney disease when delivered virtually compared with F2F. METHOD Group 1: Pre-COVID-19 lockdown: 1 January 2020–23 March 2020 CYP and their families received youth worker support F2F. Group 2: During COVID-19 lockdown: 23 March 2020–31 December 2020 youth work was delivered virtually. CYP in Groups 1 and 2 received 1:1 sessions, fun group activities and workshops. Feedback was collected on the effectiveness of these sessions. RESULTS CYP: Group 1 n = 105. Group 2 n = 639. In Group 2 there was a 102% increase/month in CYP accessing PEEER events. In both Groups, 100% said they enjoyed the sessions, would like to attend future sessions. CYP in both Groups felt part of a friendship group, said the sessions allowed them to socialize with their peers. CYP in both Groups felt more confident, more independent and good about themselves. (See Table 2.) CONCLUSION PEEER youth worker support is effective and can meet the psychosocial needs of CYP when delivered virtually and F2F. More work is needed on a hybrid approach of F2F and virtual delivery.

International audience; Disorders of the autosomal dominant polycystic kidney disease (ADPKD) spectrum are characterized by the development of kidney cysts and progressive kidney function decline. PKD1 and PKD2, encoding polycystin (PC)1 and 2, are the two major genes associated with ADPKD; other genes include IFT140, GANAB, DNAJB11, and ALG9. Genetic testing remains inconclusive in ∼7% of the families. We performed whole-exome sequencing in a large multiplex genetically unresolved (GUR) family affected by ADPKD-like symptoms and identified a monoallelic frameshift variant (c.703\₇04delCA) in ALG5. ALG5 encodes an endoplasmic-reticulum-resident enzyme required for addition of glucose molecules to the assembling N-glycan precursors. To identify additional families, we screened a cohort of 1,213 families with ADPKD-like and/or autosomal-dominant tubulointerstitial kidney diseases (ADTKD), GUR (n = 137) or naive to genetic testing (n = 1,076), by targeted massively parallel sequencing, and we accessed Genomics England 100,000 Genomes Project data. Four additional families with pathogenic variants in ALG5 were identified. Clinical presentation was consistent in the 23 affected members, with non-enlarged cystic kidneys and few or no liver cysts; 8 subjects reached end-stage kidney disease from 62 to 91 years of age. We demonstrate that ALG5 haploinsufficiency is sufficient to alter the synthesis of the N-glycan chain in renal epithelial cells. We also show that ALG5 is required for PC1 maturation and membrane and ciliary localization and that heterozygous loss of ALG5 affects PC1 maturation. Overall, our results indicate that monoallelic variants of ALG5 lead to a disorder of the ADPKD-spectrum characterized by multiple small kidney cysts, progressive interstitial fibrosis, and kidney function decline.